LIVER TRANSPLANTATION FOR THE TREATMENT OF CHILDREN WITH INHERITED METABOLIC LIVER DISEASES: SINGLE CENTER EXPERIENCE

Huashan liver transplantation. Background: Introducing the experience of living donor liver transplantation in the treatment of inherited metabolic liver diseases. Materials and Methods: A retrospective analysis of 53 children with inherited metabolic diseases from April 2015 to September 2019 in our hospital was performed. The end-stage liver disease model (PELD) and type of transplant, postoperative complications and outcomes were analyzed. Results: There were 7 cases of Wilson disease, 7 cases of Alagille syndrome (AGS), and 19 cases of progressive familial intrahepatic cholestasis (PFIC). The remaining types of diseases included glycogen storage disease (GSD), urea cycle disorder (UCD), hyperbilirubinemia, and fulminant hepatic failure due to genetic defects. Among them, 11 cases received deceased donor liver transplantation (DDLT), the remaining all received living donor liver transplantation (LDLT). 5 cases of right lobe, 4 cases of left lobe, and the rest of left lateral lobe was donated in LDLT. The average donor age (31.57 ± 1.204) years old, 2 patients with bile leakage after surgery and cured by conservative treatment finally, long-term follow-up without any other complications. The recipients were all treated with a piggy-back transplantation, the median PELD score was 19 (range1-43) and a median age of 58.4 months (± 8.7 months). There were 3 cases of perioperative death, including 1 case of pulmonary infection, 1 case of postoperative pneumothorax, 1 case of primary non-function. One patient died of pulmonary infection after discharge from hospital, and the remaining recipients survived to the present day. Postoperative complications included 5 cases of pulmonary infection, 8 cases of biliary bile leakage or stenosis, 5 cases of EBV, CMV or hepatitis B infection, and 4 cases of chylous leakage. The cumulative survival rates of recipients at 1 year, 2 years, and 3 years were 92.5%, 92.5%, and 92.5%, respectively. The cumulative survival rates of grafts at 1 year, 2 years, and 3 years were 92.5%, 92.5% and 92.5%, respectively. Conclusion: Living donor liver transplantation can achieve a better prognosis in the treatment of genetic metabolic liver diseases, but how to reduce biliary complications and how to prevent lung infections and viral infections remains a problem.